title: Urinary bladder paraganglioma in childhood: a case report and review of the literature.
abstract: Bladder paraganglioma (BP) is a rare entity and is exceedingly uncommon in childhood. Pheochromocytomas/paragangliomas are components of several hereditary cancer syndromes, and up to 30% may be associated with germ-line mutations of genes, including VHL, RET, and SDH. We present a 16-year-old female who was admitted with macroscopic hematuria and anemia. A cystoscopy demonstrated a polypoid and hemorrhagic mass arising from the floor of the bladder. She underwent a transurethral resection of clinically suspected urothelial papilloma. A histologic examination of the tumor showed large polygonal cells with eosinophilic cytoplasm, arranged in a zellballen pattern, surrounded by a fibrous network. Immunohistochemical studies showed a strong expression of neuroendocrine markers and lack of reactivity for epithelial markers. The diagnosis of BP was established; eight months later, a recurrence was observed and the patient underwent a partial cystectomy. Our case represents the 1st BP in childhood reported in the literature with absent SDHB staining by immunohistochemistry. We discuss the clinical and pathologic findings and present a review of BP in childhood.
authors.affiliation: Department of Pathology, Case Western Reserve University, Cleveland, OH, USA. olga.bohn@gmail.com
authors.forename: Olga L
authors.initials: OL
authors.lastname: Bohn
authors.forename: Edgar
authors.initials: E
authors.lastname: Pardo-Castillo
authors.forename: Mariana
authors.initials: M
authors.lastname: Fuertes-Camilo
authors.forename: Nina P
authors.initials: NP
authors.lastname: Rios-Luna
authors.forename: Andres
authors.initials: A
authors.lastname: Martinez
authors.forename: Sergio
authors.initials: S
authors.lastname: Sanchez-Sosa
mesh_terms: D000293:Adolescent; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000601:surgery; D010236:Paraganglioma, Extra-Adrenal / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D001749:Urinary Bladder Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery